Background. Acute esophageal necrosis is a rare and potentially lethal entity. The pathogenesis is multifactorial, generally presenting with symptoms of upper gastrointestinal bleeding. We present a case that presents atypically with initial esophageal perforation. Case presentation. A 46-year-old man with a history of alcoholism and cocaine use, an active smoker, and a ruptured celiac trunk aneurysm treated by embolization, who, after acute chest and epigastric pain, is diagnosed with a Stanford B thoracoabdominal aortic dissection, being repaired endovascularly by placing an aortic endoprosthesis. Due to clinical suspicion of mesenteric ischemia complicated with esophageal/gastric perforation, a postoperative tomography was performed, revealing perforation of the esophagus distal to the left pleura and ischemic cholecystitis. Transhiatal esophagectomy, cervical esophagostomy, Witzel-type decompressive gastrostomy, Witzel-type feeding jejunostomy, classic cholecystectomy, and mediastinum drainage were performed. During the postoperative period, the patient remained in critical condition, dying as a result of hypoxic encephalopathy. The histopathological study reported acute transmural esophageal ischemia. Discussion. Tissue hypoperfusion plays a dominant role in the pathogenesis of acute esophageal necrosis. Esophageal perforation is a serious complication and can occur in the early stages, with esophagectomy and deferred digestive reconstruction being the appropriate treatment. Conclusion. Ischemia is a fundamental mechanism of acute esophageal necrosis; its diagnosis must always be established in the various complications that may occur in patients with hemodynamic compromise, in order to obtain a timely treatment.